Identification of novel genetic risk factors of dilated cardiomyopathy: from canine to human.

Arrhythmia Cardiac Cardiology Companion animal Complex trait GWAS Genetics Transcriptomics

Journal

Genome medicine
ISSN: 1756-994X
Titre abrégé: Genome Med
Pays: England
ID NLM: 101475844

Informations de publication

Date de publication:
18 09 2023
Historique:
received: 09 03 2023
accepted: 17 08 2023
medline: 20 9 2023
pubmed: 19 9 2023
entrez: 18 9 2023
Statut: epublish

Résumé

Dilated cardiomyopathy (DCM) is a life-threatening heart disease and a common cause of heart failure due to systolic dysfunction and subsequent left or biventricular dilatation. A significant number of cases have a genetic etiology; however, as a complex disease, the exact genetic risk factors are largely unknown, and many patients remain without a molecular diagnosis. We performed GWAS followed by whole-genome, transcriptome, and immunohistochemical analyses in a spontaneously occurring canine model of DCM. Canine gene discovery was followed up in three human DCM cohorts. Our results revealed two independent additive loci associated with the typical DCM phenotype comprising left ventricular systolic dysfunction and dilatation. We highlight two novel candidate genes, RNF207 and PRKAA2, known for their involvement in cardiac action potentials, energy homeostasis, and morphology. We further illustrate the distinct genetic etiologies underlying the typical DCM phenotype and ventricular premature contractions. Finally, we followed up on the canine discoveries in human DCM patients and discovered candidate variants in our two novel genes. Collectively, our study yields insight into the molecular pathophysiology of DCM and provides a large animal model for preclinical studies.

Sections du résumé

BACKGROUND
Dilated cardiomyopathy (DCM) is a life-threatening heart disease and a common cause of heart failure due to systolic dysfunction and subsequent left or biventricular dilatation. A significant number of cases have a genetic etiology; however, as a complex disease, the exact genetic risk factors are largely unknown, and many patients remain without a molecular diagnosis.
METHODS
We performed GWAS followed by whole-genome, transcriptome, and immunohistochemical analyses in a spontaneously occurring canine model of DCM. Canine gene discovery was followed up in three human DCM cohorts.
RESULTS
Our results revealed two independent additive loci associated with the typical DCM phenotype comprising left ventricular systolic dysfunction and dilatation. We highlight two novel candidate genes, RNF207 and PRKAA2, known for their involvement in cardiac action potentials, energy homeostasis, and morphology. We further illustrate the distinct genetic etiologies underlying the typical DCM phenotype and ventricular premature contractions. Finally, we followed up on the canine discoveries in human DCM patients and discovered candidate variants in our two novel genes.
CONCLUSIONS
Collectively, our study yields insight into the molecular pathophysiology of DCM and provides a large animal model for preclinical studies.

Identifiants

pubmed: 37723491
doi: 10.1186/s13073-023-01221-3
pii: 10.1186/s13073-023-01221-3
pmc: PMC10506233
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

73

Subventions

Organisme : Department of Health
Pays : United Kingdom

Investigateurs

Carsten Daub (C)
César L Araujo (CL)
Ileana B Quintero (IB)
Kaisa Kyöstilä (K)
Maria Kaukonen (M)
Meharji Arumilli (M)
Riika Sarviaho (R)
Jenni Puurunen (J)
Sini Sulkama (S)
Sini Karjalainen (S)
Antti Sukura (A)
Pernilla Syrjä (P)
Niina Airas (N)
Henna Pekkarinen (H)
Ilona Kareinen (I)
Hanna-Maaria Javela (HM)
Anna Knuuttila (A)
Heli Nordgren (H)
Karoliina Hagner (K)
Tarja Pääkkönen (T)
Antti Iivanainen (A)
Kaarel Krjutskov (K)
Sini Ezer (S)
Auli Saarinen (A)
Shintaro Katayama (S)
Masahito Yoshihara (M)
Abdul Kadir Mukarram (AK)
Rasha Fahad Aljelaify (RF)
Fiona Ross (F)
Amitha Raman (A)
Irene Stevens (I)
Oleg Gusev (O)
Danika Bannasch (D)
Jeffrey J Schoenebeck (JJ)

Informations de copyright

© 2023. BioMed Central Ltd., part of Springer Nature.

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Auteurs

Julia E Niskanen (JE)

Department of Medical and Clinical Genetics, University of Helsinki, Haartmaninkatu 8, 00290, Helsinki, Finland.
Department of Veterinary Biosciences, University of Helsinki, Agnes Sjöbergin katu 2, 00790, Helsinki, Finland.
Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland.

Åsa Ohlsson (Å)

Department of Animal Breeding and Genetics, Swedish University of Agricultural Sciences, Uppsala, Sweden.

Ingrid Ljungvall (I)

Department of Clinical Sciences, Swedish University of Agricultural Sciences, Uppsala, Sweden.

Michaela Drögemüller (M)

Institute of Genetics, Vetsuisse Faculty, University of Bern, Bern, 3001, Switzerland.

Robert F Ernst (RF)

Department of Genetics, University Medical Centre Utrecht, Utrecht University, Utrecht, The Netherlands.

Dennis Dooijes (D)

Department of Genetics, University Medical Centre Utrecht, Utrecht University, Utrecht, The Netherlands.

Hanneke W M van Deutekom (HWM)

Department of Genetics, University Medical Centre Utrecht, Utrecht University, Utrecht, The Netherlands.

J Peter van Tintelen (JP)

Department of Genetics, University Medical Centre Utrecht, Utrecht University, Utrecht, The Netherlands.

Christian J B Snijders Blok (CJB)

Department of Cardiology, Division Heart & Lungs, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands.
Regenerative Medicine Centre Utrecht, University of Utrecht, Utrecht, The Netherlands.

Marion van Vugt (M)

Department of Cardiology, Division Heart & Lungs, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands.

Jessica van Setten (J)

Department of Cardiology, Division Heart & Lungs, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands.

Folkert W Asselbergs (FW)

Amsterdam University Medical Centers, Department of Cardiology, University of Amsterdam, Amsterdam, The Netherlands.
Health Data Research UK and Institute of Health Informatics, University College London, London, UK.

Aleksandra Domanjko Petrič (AD)

Small Animal Clinic, Veterinary Faculty, University of Ljubljana, Ljubljana, 1000, Slovenia.

Milla Salonen (M)

Department of Medical and Clinical Genetics, University of Helsinki, Haartmaninkatu 8, 00290, Helsinki, Finland.
Department of Veterinary Biosciences, University of Helsinki, Agnes Sjöbergin katu 2, 00790, Helsinki, Finland.
Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland.

Sruthi Hundi (S)

Department of Medical and Clinical Genetics, University of Helsinki, Haartmaninkatu 8, 00290, Helsinki, Finland.
Department of Veterinary Biosciences, University of Helsinki, Agnes Sjöbergin katu 2, 00790, Helsinki, Finland.
Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland.

Matthias Hörtenhuber (M)

Department of Biosciences and Nutrition, Karolinska Institutet, Huddinge, Sweden.

Juha Kere (J)

Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland.
Department of Biosciences and Nutrition, Karolinska Institutet, Huddinge, Sweden.
Research Programs Unit, Stem Cells and Metabolism Research Program, University of Helsinki, Helsinki, Finland.

W Glen Pyle (WG)

Department of Biomedical Sciences, University of Guelph, Guelph, ON, Canada.
IMPART Investigator Team Canada, Dalhousie Medicine, Saint John, NB, Canada.

Jonas Donner (J)

Wisdom Panel Research Team, Wisdom Panel, Kinship, Helsinki, Finland.

Alex V Postma (AV)

Department of Human Genetics, Amsterdam University Medical Center, Amsterdam, The Netherlands.
Department of Medical Biology, Amsterdam University Medical Center, Amsterdam, The Netherlands.

Tosso Leeb (T)

Institute of Genetics, Vetsuisse Faculty, University of Bern, Bern, 3001, Switzerland.

Göran Andersson (G)

Department of Animal Breeding and Genetics, Swedish University of Agricultural Sciences, Uppsala, Sweden.

Marjo K Hytönen (MK)

Department of Medical and Clinical Genetics, University of Helsinki, Haartmaninkatu 8, 00290, Helsinki, Finland.
Department of Veterinary Biosciences, University of Helsinki, Agnes Sjöbergin katu 2, 00790, Helsinki, Finland.
Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland.

Jens Häggström (J)

Department of Clinical Sciences, Swedish University of Agricultural Sciences, Uppsala, Sweden.

Maria Wiberg (M)

Department of Equine and Small Animal Medicine, University of Helsinki, Helsinki, Finland.

Jana Friederich (J)

LMU Small Animal Clinic, Ludwig Maximilians University of Munich, Munich, Germany.

Jenny Eberhard (J)

LMU Small Animal Clinic, Ludwig Maximilians University of Munich, Munich, Germany.

Magdalena Harakalova (M)

Department of Cardiology, Division Heart & Lungs, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands.
Regenerative Medicine Centre Utrecht, University of Utrecht, Utrecht, The Netherlands.

Frank G van Steenbeek (FG)

Department of Cardiology, Division Heart & Lungs, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands.
Regenerative Medicine Centre Utrecht, University of Utrecht, Utrecht, The Netherlands.
Department of Clinical Sciences, Faculty of Veterinary Medicine, Utrecht University, Yalelaan 108, Utrecht, 3584 CM, The Netherlands.

Gerhard Wess (G)

LMU Small Animal Clinic, Ludwig Maximilians University of Munich, Munich, Germany.

Hannes Lohi (H)

Department of Medical and Clinical Genetics, University of Helsinki, Haartmaninkatu 8, 00290, Helsinki, Finland. hannes.lohi@helsinki.fi.
Department of Veterinary Biosciences, University of Helsinki, Agnes Sjöbergin katu 2, 00790, Helsinki, Finland. hannes.lohi@helsinki.fi.
Folkhälsan Research Center, Haartmaninkatu 8, P.O.Box 63, 00290, Helsinki, Finland. hannes.lohi@helsinki.fi.

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